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Atypical Ewing Sarcoma

MedGen UID:
785386
•Concept ID:
C3698122
•
Neoplastic Process
Synonyms: Atypical Ewing's sarcoma; Large Cell Ewing Sarcoma; Large cell Ewing sarcoma of bone; Large cell Ewing's sarcoma of bone
SNOMED CT: Large cell Ewing sarcoma of bone (698200003); Large cell Ewing's sarcoma of bone (698200003); Atypical Ewing's sarcoma (698200003)

Definition

Ewing sarcoma characterized by the presence of large malignant cells with prominent nucleoli and irregular contours. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAtypical Ewing Sarcoma

Professional guidelines

PubMed

Small cell osteosarcoma of bone: an immunohistochemical study with differential diagnostic considerations.
Devaney K, Vinh TN, Sweet DE
Hum Pathol 1993 Nov;24(11):1211-25. doi: 10.1016/0046-8177(93)90218-6. PMID: 7503935

Recent clinical studies

Etiology

"Cyst" on the forearm of a 28-year-old female: Case report of a CIC-rearranged sarcoma.
Lehane F, Tsikleas G, Bettington A, Limarporn K, Wilkinson L, Lehane K
J Cutan Pathol 2019 Aug;46(8):599-602. Epub 2019 May 6 doi: 10.1111/cup.13478. PMID: 30989672
Sarcomas With CIC-rearrangements Are a Distinct Pathologic Entity With Aggressive Outcome: A Clinicopathologic and Molecular Study of 115 Cases.
Antonescu CR, Owosho AA, Zhang L, Chen S, Deniz K, Huryn JM, Kao YC, Huang SC, Singer S, Tap W, Schaefer IM, Fletcher CD
Am J Surg Pathol 2017 Jul;41(7):941-949. doi: 10.1097/PAS.0000000000000846. PMID: 28346326Free PMC Article
Correlation of Classic and Molecular Cytogenetic Alterations in Soft-Tissue Sarcomas: Analysis of 46 Tumors With Emphasis on Adipocytic Tumors and Synovial Sarcoma.
Rao UN, Cieply K, Sherer C, Surti U, Gollin SM
Appl Immunohistochem Mol Morphol 2017 Mar;25(3):168-177. doi: 10.1097/PAI.0000000000000294. PMID: 26808135
Atypical Ewing sarcoma breakpoint region 1 fluorescence in-situ hybridization signal patterns in bone and soft tissue tumours: diagnostic experience with 135 cases.
Vargas AC, Selinger CI, Satgunaseelan L, Cooper WA, Gupta R, Stalley P, Brown W, Soper J, Schatz J, Boyle R, Thomas DM, Tattersall MH, Bhadri VA, Maclean F, Bonar SF, Scolyer RA, Karim RZ, McCarthy SW, Mahar A, O'Toole SA
Histopathology 2016 Dec;69(6):1000-1011. Epub 2016 Sep 21 doi: 10.1111/his.13031. PMID: 27385661
Morphologic and immunophenotypic diversity in Ewing family tumors: a study of 66 genetically confirmed cases.
Folpe AL, Goldblum JR, Rubin BP, Shehata BM, Liu W, Dei Tos AP, Weiss SW
Am J Surg Pathol 2005 Aug;29(8):1025-33. PMID: 16006796

Diagnosis

Important Recently Characterized Non-Ewing Small Round Cell Tumors.
Carter CS, Patel RM
Surg Pathol Clin 2019 Mar;12(1):191-215. Epub 2018 Dec 4 doi: 10.1016/j.path.2018.10.008. PMID: 30709443
Update on Families of Round Cell Sarcomas Other than Classical Ewing Sarcomas.
Le Loarer F, Pissaloux D, Coindre JM, Tirode F, Vince DR
Surg Pathol Clin 2017 Sep;10(3):587-620. Epub 2017 Jun 20 doi: 10.1016/j.path.2017.04.002. PMID: 28797504
Correlation of Classic and Molecular Cytogenetic Alterations in Soft-Tissue Sarcomas: Analysis of 46 Tumors With Emphasis on Adipocytic Tumors and Synovial Sarcoma.
Rao UN, Cieply K, Sherer C, Surti U, Gollin SM
Appl Immunohistochem Mol Morphol 2017 Mar;25(3):168-177. doi: 10.1097/PAI.0000000000000294. PMID: 26808135
Atypical Ewing sarcoma breakpoint region 1 fluorescence in-situ hybridization signal patterns in bone and soft tissue tumours: diagnostic experience with 135 cases.
Vargas AC, Selinger CI, Satgunaseelan L, Cooper WA, Gupta R, Stalley P, Brown W, Soper J, Schatz J, Boyle R, Thomas DM, Tattersall MH, Bhadri VA, Maclean F, Bonar SF, Scolyer RA, Karim RZ, McCarthy SW, Mahar A, O'Toole SA
Histopathology 2016 Dec;69(6):1000-1011. Epub 2016 Sep 21 doi: 10.1111/his.13031. PMID: 27385661
BCOR-CCNB3 (Ewing-like) sarcoma: a clinicopathologic analysis of 10 cases, in comparison with conventional Ewing sarcoma.
Puls F, Niblett A, Marland G, Gaston CL, Douis H, Mangham DC, Sumathi VP, Kindblom LG
Am J Surg Pathol 2014 Oct;38(10):1307-18. doi: 10.1097/PAS.0000000000000223. PMID: 24805859

Prognosis

Sarcomas With CIC-rearrangements Are a Distinct Pathologic Entity With Aggressive Outcome: A Clinicopathologic and Molecular Study of 115 Cases.
Antonescu CR, Owosho AA, Zhang L, Chen S, Deniz K, Huryn JM, Kao YC, Huang SC, Singer S, Tap W, Schaefer IM, Fletcher CD
Am J Surg Pathol 2017 Jul;41(7):941-949. doi: 10.1097/PAS.0000000000000846. PMID: 28346326Free PMC Article
Correlation of Classic and Molecular Cytogenetic Alterations in Soft-Tissue Sarcomas: Analysis of 46 Tumors With Emphasis on Adipocytic Tumors and Synovial Sarcoma.
Rao UN, Cieply K, Sherer C, Surti U, Gollin SM
Appl Immunohistochem Mol Morphol 2017 Mar;25(3):168-177. doi: 10.1097/PAI.0000000000000294. PMID: 26808135
BCOR-CCNB3 (Ewing-like) sarcoma: a clinicopathologic analysis of 10 cases, in comparison with conventional Ewing sarcoma.
Puls F, Niblett A, Marland G, Gaston CL, Douis H, Mangham DC, Sumathi VP, Kindblom LG
Am J Surg Pathol 2014 Oct;38(10):1307-18. doi: 10.1097/PAS.0000000000000223. PMID: 24805859

Clinical prediction guides

Atypical Ewing sarcoma breakpoint region 1 fluorescence in-situ hybridization signal patterns in bone and soft tissue tumours: diagnostic experience with 135 cases.
Vargas AC, Selinger CI, Satgunaseelan L, Cooper WA, Gupta R, Stalley P, Brown W, Soper J, Schatz J, Boyle R, Thomas DM, Tattersall MH, Bhadri VA, Maclean F, Bonar SF, Scolyer RA, Karim RZ, McCarthy SW, Mahar A, O'Toole SA
Histopathology 2016 Dec;69(6):1000-1011. Epub 2016 Sep 21 doi: 10.1111/his.13031. PMID: 27385661
BCOR-CCNB3 (Ewing-like) sarcoma: a clinicopathologic analysis of 10 cases, in comparison with conventional Ewing sarcoma.
Puls F, Niblett A, Marland G, Gaston CL, Douis H, Mangham DC, Sumathi VP, Kindblom LG
Am J Surg Pathol 2014 Oct;38(10):1307-18. doi: 10.1097/PAS.0000000000000223. PMID: 24805859
Comparative analysis of routine histology, immunohistochemistry, reverse transcriptase polymerase chain reaction, and fluorescence in situ hybridization in diagnosis of Ewing family of tumors.
Jambhekar NA, Bagwan IN, Ghule P, Shet TM, Chinoy RF, Agarwal S, Joshi R, Amare Kadam PS
Arch Pathol Lab Med 2006 Dec;130(12):1813-8. doi: 10.5858/2006-130-1813-CAORHI. PMID: 17149955

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